Invitrogen™ HMBS Polyclonal Antibody

Rabbit Polyclonal Antibody

Brand:  Invitrogen™ PA595924

Product Code. 16588761

Description

Immunogen sequence: MSGNGNAAAT AEENSPKMRV IRVGTRKSQL ARIQTDSVVA TLKASYPGLQ FEIIAMSTTG DKILDTALSK IGEKSLFTKE LEHALEKNEV DLVVHSLKDL PTVLPPGFTI GAICKRENPH DAVVFHPKFV GKTLETLPEK SVVGTSSLRR AAQLQRKFPH LEFRSIRGNL NTRLRKLDEQ QEFSAIILAT AGLQRMGWHN RVGQILHPEE CMYAVGQGAL GVEVRAKDQD ILDLVGVLHD PETLLRCIAE RAFLRHLEGG CSVPVAVHTA MKDGQLYLTG GVWSLDGSDS IQETMQATIH VPAQHEDGPE DDPQLVGITA RNIPRGPQLA AQNLGISLAN LLLSKGAKNI LDVARQLNDA H; Positive Samples: HeLa, Raji, Mouse spleen; Cellular Location: Cytoplasm.

PBGD (porphobilinogen deaminase), also designated hydroxymethylbilane synthase, is a cytoplasmic enzyme found in the heme synthesis pathway. PBGD belongs to the HMBS (hydroxymethylbilane synthase) family. Deficiency of PBGD causes errors in pyrrole metabolism, which in turn leads to an inherited autosomal disorder called acute intermittent porphyria (AIP). AIP is characterized by acute attacks of neurological dysfunctions with hypertension, tachycardia, peripheral neurologic disturbances, abdominal pain and excessive amounts of aminolevulinic acid and porphobilinogen in the urine.

Specifications

• Antigen: HMBS
• Classification: Polyclonal
• Conjugate: Unconjugated
• Gene: Hmbs
• Gene Alias: alternative name: porphobilinogen deaminase; HEM3; hemC; HMBS; Hydroxymethylbilane synthase; PBG D; PBGD; PBG-D; PORC; Porphobilinogen deaminase; porphyria, acute; Chester type; pre-uroporphyrinogen synthase; similar to Porphobilinogen deaminase (Hydroxymethylbilane synthase) (HMBS) (Pre-uroporphyrinogen synthase) (PBG-D); T25658; Ups; uroporphyrinogen I synthase; uroporphyrinogen I synthetase; URO-S; Uros1
• Host Species: Rabbit
• Purification Method: Affinity Chromatography
• Regulatory Status: RUO
• Gene ID (Entrez): 15288, 3145
• Content And Storage: -20°C, Avoid Freeze/Thaw Cycles
• Form: Liquid

• Applications: ELISA, Western Blot
• Concentration: 6.93 mg/mL
• Formulation: PBS with 50% glycerol and 0.02% sodium azide; pH 7.3
• Gene Accession No.: P08397, P22907
• Gene Symbols: Hmbs
• Immunogen: Recombinant protein (or fragment).
• Quantity: 100 μL
• Primary or Secondary: Primary
• Target Species: Human, Mouse
• Product Type: Antibody
• Isotype: IgG